Flashcards in congenital heart disease Deck (16):
What are the right-to-left shunts?
truncus arteriosus (1 vessel), transposition (2 switched vessels), tricuspid atresia (tri), tetralogy of fallot (4), TAPVR (total anomalous pulmonary venous return)
persistant truncus arteriosus
failure of the truncus to divide into pulm trunk and aorta. most pts also have a VSD
tx for most right to left shunts; coloring
surgical correction and/or maintenance of a PDA
these babies are cyanotic.
transposition of the great vessels
Associated with maternal diabetes!!!
aorta leaves the RV (anterior) and the pulmonary trunk leaves the LV (posterior). this separates the systemic and pulmonary circulations. it is incompatible with life unless a shunt is present to allow mixing of blood (VSD, PDA, patent forament ovale). due to failure of the aorticopulmonary septum to spiral. most infants die in first few months without surgical intervention
absence of the tricuspid and hypoplastic RV. requires both ASD and VSD for viability
tetrology of fallot
1. pulmonary artery stenosis
2. right ventricular hypertrophy- looks like a boot.
3. overriding aorta
4. ventricular septal defect
forces right to left flow across the VSD and causes early cyanotic spells.
how does squatting affect the tetrology of fallot
squatting: increased systemic vascular resistance, decreases right to left shunt, and improves cyanosis.
what is TAPVR
total anomalous pulmonary venous return.
pulmonary vein drains to right heart. this is associated with ASD and sometimes PDA to allow for right-to-left shunting to maintain CO.
causes of late cyanosis
1. left-to-right shunt causes increased pulmonary flow --> pulm remodeling and pulm arteriole HTN. Right ventricular HTN occus to compensate, and the shunt becomes right to left. this causes late cyanosis, clubbing, and polycythemia.
most common left to right defect. associated with fetal alcohol syndrome. asymmptomatic at birth, and may or may not manifest later. if very large, it may lead to LV overload and heart failure.
defect in inter-atrial septum. loud S1. wide, fixed, split S2. usually occurs at septum secundum; septum primum defects occur with other abnormalities (and are associated with Down syndrome). distinct from PFO in that the septa tissue is missing, not unfused.
associated with a continuous machine like murmur. patency is maintained by PGE synthesis and low O2. it can eventually result in late cyanosis of the lower extremities. can cause progressive RVG and/or LVH and/or heart failure
What other conditions are associated with coarctation of the aorta?
bicuspid aortic valve
infantile coarctation of the aorta
narrowing proximal to the insertion of the ductus arteriousus. associated with Turner syndrome. may present with closure of the ductus arteriosus.